POS1293 STRAIN OF THE RIGHT VENTRICLE AS A TOOL OF EARLY DETECTION OF PULMONARY VASCULATURE ABNORMALITY IN SYSTEMIC SCLEROSIS
Authors: V. Codullo, B. L. Palermo, M. Acquaro, M. Lia, G. Zanframundo, L. Scelsi, A. Greco, S. Schirinzi, L. Cavagna, C. Montecucco, S. Ghio
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Patients with systemic Sclerosis (SSc) are at risk of developing pulmonary hypertension (PH) of different etiologies. Two-dimensional and Doppler echocardiography is the reference screening tool and it is recommended yearly even in the absence of symptoms; however, elevated values of systolic pulmonary artery pressure (sPAP) are not detectable at rest in the early phases of the disease. <h3>Objectives</h3> The aim of the study was to determine whether early signs of right ventricular or right atrial dysfunction at speckle-tracking echocardiography might identify patients who are going to develop PH during follow-up. <h3>Methods</h3> We studied a consecutive cohort of 113 patients with a diagnosis of SSc according to the 2013 ACR/EULAR SSc classification criteria <sup>[1]</sup>, undergoing screening for PH and who were followed-up regularly at a third-level centre of reference for SSc. All patients underwent both the baseline echocardiographic examinations and at least one echocardiographic examination during follow-up in the referral center. Standard 2-D and Doppler echocardiography was performed with a Vivid 7 or Vivid E9 ultrasound system (GE Medical Systems, Norway); images and clips were saved for offline analysis on a GE workstation (EchoPAC PC SW). Clinical, laboratory and radiographical parameters, standard in the care of SSc, such as presence of Interstitial lung disease, left-heart disease (LHD), pulmonary function respiratory tests and biomarkers as BNP were concomitantly recorded for each patient. The DETECT step 1 and 2 values were calculated according to Coghlan JG et al <sup>[2]</sup>. When indicated according to guidelines, right heart catheterization to diagnose PH was performed. Statistical analysis was performed using SPSS by IBM for mac (v13). <h3>Results</h3> The study enrolled 93 females and 20 males, mean age was 58±13 yrs, median disease duration was 88 months (IQR 42-168); 86 (76%) patients had limited cutaneous involvement, 21 had a diffuse disease (18,6%) and 5 (4,4%) had no skin involvement at all. At baseline, anticentromere and antitopoisomerase antibodies were present in 38 (33,6%) and in 35 (31%) patients respectively. A clinically significant ILD was present in 17 (15%) of patients of the cohort at baseline. During follow-up (44±24 months), 10 patients were diagnosed with PH (overall incidence of 8.4%) which was classified as Group 1 PH in 4 cases (40%), Group 2 PH in 1 case (10%) in Group 3 PH in 5 cases (50%). Baseline standard echocardiographic parameters including the estimated sPAP and DETECT scores for step 1 and step 2 were similar in patients who developed PH and those who did not. However, peak longitudinal right ventricular (RV) strain (RVPLS) values were significantly lower in patients who subsequently developed PH (42,13 ± 12,21 vs 33,57 ± 11,52, p=0,019). In addition, PLS values tended to decrease over time and this decrease significantly predicted PH of any etiology (HR 0.94, 0,88-0,99; p=0,049). <h3>Conclusion</h3> In an SSc cohort of patients at risk of developing PH, adding the evaluation of 2D-Speckle-tracking echocardiography (STE) analysis, and in particular PLS of the RV, might intercept at an early stage an alteration of the right heart to aberrantly adapt to the pulmonary vasculature overload in different extrinsic pathological settings (ILD, LHD) and also in the presence of an intrinsic pulmonary vasculopathy. <h3>References</h3> [1]van den Hogen et al. Ann Rheum Dis 2013 [2]Coghlan JG et al. Ann Rheum Dis 2014 <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.